Zhang Wei-xi, Zhang Hai-lin, Li Chang-chong, Luo Yun-chun, Cheng Jian-min, Huang Lei, Bai Guang-hui
Department of Respiratory Diseases, Yuying Children's Hospital Affiliated to Wenzhou Medical College, Wenzhou 325027, China.
Zhonghua Er Ke Za Zhi. 2009 Jun;47(6):436-40.
To explore clinical characteristics, radiographic findings and diagnostic methods of patients with congenital malformations of respiratory system for enhancing the diagnosis of congenital malformations of respiratory system in children.
Totally 234 patients with congenital malformations of respiratory system were chosen from the inpatient department of Yuying Children's Hospital Affiliated to Wenzhou Medical College from July 2003 to June 2008. The clinical presentations and radiographic findings of these children were analyzed.
Of the 234 patients with congenital malformations of respiratory system, the age at diagnosis was between the first day and 14 years of age, mean age was 1.12 years. The main symptoms were persistent laryngeal stridor, recurrent wheezing, recurrent respiratory tract infections and dyspnea. Through the use of chest X-ray, spiral CT 3D reconstructions, fiberoptic bronchoscopy and other laboratory techniques, 213 cases were diagnosed as having single malformation and 21 cases were found to have multiple malformations. Of the 213 cases with single malformation, 97 cases had laryngeal malformation (congenital laryngeal stridor in 90 cases, congenital laryngeal webs in 5 cases and congenital laryngeal cyst in 2 cases), 35 cases had tracheal-bronchial malformation (congenital tracheobronchial stenosis in 17 cases, congenital abnormal bronchial origin in 7 cases, tracheobronchomalacia in 10 cases and tracheoesophageal fistula in 1 case), 43 cases had lung malformation (pulmonary sequestration in 5 cases, congenital lung cysts in 22 cases, congenital lobar emphysema in 1 case, agenesis of lung and hypoplasia of lung in 8 cases and congenital cystic adenomatoid malformation in 7 cases), 38 cases had diaphragm malformation, 28 cases had congenital tracheal-bronchial stenosis as confirmed by spiral CT 3D reconstructions and fiberoptic bronchoscopy. Ten cases with congenital abnormal bronchial origin were diagnosed with spiral CT 3D reconstructions. Laryngeal stridor and tracheobronchomalacia were diagnosed by fiberoptic laryngoscope and fiberoptic bronchoscopy. The accuracy rates of preoperative diagnosis through clinical and radiographic examinations of 37 cases with lung malformation and 36 cases with diaphragm malformation were 83.78% and 91.67%.
Congenital malformations of respiratory system are a group of diseases that are important for pediatric respiratory clinicians. Congenital malformations of respiratory system should be considered in children with persistent laryngeal stridor, recurrent wheezing, recurrent respiratory tract infections and dyspnea. The radiographic examination and respiratory endoscope play important roles in the diagnosis of congenital malformations of respiratory system.
探讨呼吸系统先天性畸形患儿的临床特征、影像学表现及诊断方法，以提高儿童呼吸系统先天性畸形的诊断水平。
选取2003年7月至2008年6月温州医学院附属育英儿童医院住院部收治的234例呼吸系统先天性畸形患儿，分析其临床表现及影像学检查结果。
234例呼吸系统先天性畸形患儿诊断年龄为出生第1天至14岁，平均年龄1.12岁。主要症状为持续性喉喘鸣、反复喘息、反复呼吸道感染及呼吸困难。通过胸部X线、螺旋CT三维重建、纤维支气管镜等检查手段，确诊单发畸形213例，多发畸形21例。213例单发畸形中，喉畸形97例（先天性喉喘鸣90例、先天性喉蹼5例、先天性喉囊肿2例），气管支气管畸形35例（先天性气管支气管狭窄17例、先天性支气管起源异常7例、气管支气管软化10例、气管食管瘘1例），肺畸形43例（肺隔离症5例、先天性肺囊肿22例、先天性大叶性肺气肿1例、肺不发育及肺发育不全8例、先天性囊性腺瘤样畸形7例），膈肌畸形38例，螺旋CT三维重建及纤维支气管镜确诊先天性气管支气管狭窄28例，螺旋CT三维重建确诊先天性支气管起源异常10例，纤维喉镜及纤维支气管镜确诊喉喘鸣及气管支气管软化。37例肺畸形及36例膈肌畸形术前临床及影像学检查诊断准确率分别为83.78%和91.67%。
呼吸系统先天性畸形是儿科呼吸科医师应重点关注的一组疾病。对于有持续性喉喘鸣、反复喘息、反复呼吸道感染及呼吸困难的患儿应考虑呼吸系统先天性畸形。影像学检查及呼吸内镜在呼吸系统先天性畸形诊断中具有重要作用。
